Disorders of amino acid metabolism 1.cuni.cz

[Pages:82]Disorders of amino acid metabolism



General comments on AA metabolis Urea cycle disorders Disorders of aromatic AA metabolism Disorders of branched chain AA metabolism Disorders of sulfur metabolism Other disorders of AA metabolism

~ 400 g



Energy stores

Energy store

Glycogen Glycogen Glucose Triacylglycerols Proteins

tissue

liver muscle blood fat muscle

amount energy

(g)

(kj) (kcal)

70

1176

280

120

2016

480

20

336

80

15 000

567 000 135 000

6000

100 800 24 000







Protein requirements

Protein intake



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